1
by, or produces, a permanent or prolonged derangement of neu- rologic functions. We discuss the implications that this new perspective has, both from the epileptologic and neuropsycho- logic points of view. 192. LONG LATENCY EVENT-RELATED POTEN- TIALS IN EVALUATION OF BEHAVIORAL PROB- LEMS IN GILLES DE LA TOURETTE SYNDROME Tianzhu Wang and Zhijie Cui, Dalian, China Gilles de la Tourette syndrome (GTS) is a hyperactive disorder characterized by both motor and vocal tics. It may also be ac- companied by a variety of behavioral problems. The Achenbach Child Behavioral Check List (A-CBCL) and the endogenous auditory P300 event-related potential have been used to evaluate the behavioral disorder in children with GTS. We found that the behavioral disorders include schizoid or anxious, depressed, un- communicative, obsessive-compulsive, somatic complaint, so- cial withdrawal, hyperactive, aggressive, and delinquent behav- ioral syndromes. The abnormal rate is 75%. Among behavioral disorder syndromes, obsessive-compulsive is highest. The P300 latency in children with GTS appeared to be more prolonged than that in normal children; the abnormal rate is 56%. Our result was analyzed by multiple liner regression. The P300 latency has a perfect positive correlation with the obsessive- compulsive syndrome. The P300 latency compared to A-CBCL showed 81% in agreement with the independent assessment of a behavioral disorder. A conclusion of our study can be drawn that a combination of A-CBCL and P300 latency is sensitive in the evaluation of behavioral disorders in children with GTS; the P300 latency may be the preferable and objective one. 193. SIGNIFICANCE AND MEASUREMENT OF MYELIN BASIC PROTEIN ANTIBODY IN CSF FROM CHILDREN WITH CNS DISEASES Chen Ling and Wang Shu-xue, Changchun, China Myelin basic protein (MBP) as an antigen was extracted from normal human brain white tissue. The level of MBP antibody (MBP-Ab) in CSF was measured with enzyme-linked immuno- sorbent assay in children with CNS diseases. In Guillain-Barr6 syndrome (GBS) the level of MBP-Ab was significantly higher than normal controls (P < .01). So it is useful in the diagnosis of GBS and contributes to the assessment of the GBS course. The study also showed that the level of MBP-Ab in children with tuberculous meningitis was significantly higher than in purulent meningitis, viral encephalitis, and a normal control group. The conjecturing measurement of MBP-Ab in CSF could be a refer- ence index for the differential diagnosis of TB meningitis. 194. PROGRESSIVE IDIOPATHIC LEVODOPA-RE- SPONSIVE DYSTONIA WITH DIURNAL FLUCTUA- TION Johan F. Schoeman, Cape Town, South Africa Since the description of the syndrome of "hereditary progressive dystonia with marked diurnal fluctuation" by Segawa et al., sporadic cases have been reported. We report the first case of Segawa-type dystonia from the African continent and demon- strate the typical diurnal fluctuation of symptoms and the prompt response to levodopa by means of a video recording. The patient is a 5-year-old boy of mixed race of unrelated par- ents. Birth and developmental milestones were normal and his medical history, including family history, was negative. At the age of 21/2 years he presented at a teaching hospital with an unsteady gait of 6 months duration. On examination he was found to "go stifP' when walking and tended to invert his left foot. Gowers' sign was positive. The formal neurologic examin- ation, however, was completely normal and a provisional diag- nosis of a "myopathy" was made. Blood tests (including CBC, Astrup, lactate, pyruvate, liver function tests, CK), urine (amino and organic acids), CSF (chemistry and culture), electrophysio- logic tests (EEG, VER, ERG, EMG, nerve conduction) and neu- roimaging (cranial CT, spinal MRI) were all negative. A muscle biopsy showed nonspecific changes. The patient's gait deterior- ated over the next 21/2 years and when first seen by us at age 5 years he could still walk a few steps in the morning but not at all by late afternoon. Walking was characterized by progressively shorter steps and increased tone of the legs, which invariably led to falling over. Apart from appearing depressed, the formal neu- rologic examination was normal. Treatment was started with Sinemet (187 mg levodopa and 18.7 mg carbidopa) and within one day the patient was able to walk and even regained the ability to run. A striking improvement of mood also occurred. Reference: [1] Segawa M, Hosaka A, Miyagawa, et al. Hered- itary progressive dystonia with marked diurnal fluctuation. Adv Neurol 1976;14:215-33. 195. CORPOCALLOSOTOMY IN INTRACTABLE EPI- LEPSY Ledia Troncoso, Fernando Novoa, Arturo Zuleta, and Sergio Valenzuela, Santiago, Chile About one-quarter of epileptic patients cannot achieve an ade- quate control of seizures, even when available polytherapy is administered. Several surgical methods have been proposed as an alternative treatment in some patients. Central corpocalloso- tomy has proved to be useful in controlling generalized atonic, tonic, and tonic-clonic seizures. Although reports are mainly referred to intellectually normal subjects, recent data suggest benefits in mentally retarded patients as well. We report the results of central corpocallosotomy in 16 patients (9 girls, 7 boys) with a mean age of 12.4 years (S.D.: 4.2), suffering from mental retardation and intractable epilepsy. All had daily atonic episodes (X: 9.5, S.D.: 7.4) and 2-6 other types of seizures, for 3-5 years, and which markedly affected their quality of life. The follow-up of these patients, with a mean of 20.4 months (S.D.: 7.6, range: 8-34) after surgical procedure was performed, shows a highly significant effect on atonic and tonic-clonic seizures, which disappeared in 14 of 16 and 8 of 14 patients, respectively. A significant decrease in tonic, complex partial, myoclonic sei- zures and absence was also observed. At the same time, parents reported improvements in quality of life, expressed as a higher level of autonomy and increased social interaction. 196. OBJECTIVE MEASURE OF TREATMENT OUT- COME IN EPILEPSY Jorge Eslava-Cobos, Bogot& Colombia Shofer and Temkin [1] presented a proposal for the determina- tion of seizure frequency in terms of time to (X) seizure. Using that general approach we have devised the following formula: PEDIATRIC NEUROLOGY Vol. 8 No. 5 393

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Page 1: Significance and measurement of myelin basic protein antibody in CSF from children with CNS diseases

by, or produces, a permanent or prolonged derangement of neu- rologic functions. We discuss the implications that this new perspective has, both from the epileptologic and neuropsycho- logic points of view.

192. LONG LATENCY EVENT-RELATED POTEN- TIALS IN EVALUATION OF BEHAVIORAL PROB- LEMS IN GILLES DE LA TOURETTE SYNDROME Tianzhu Wang and Zhijie Cui, Dalian, China

Gilles de la Tourette syndrome (GTS) is a hyperactive disorder characterized by both motor and vocal tics. It may also be ac- companied by a variety of behavioral problems. The Achenbach Child Behavioral Check List (A-CBCL) and the endogenous auditory P300 event-related potential have been used to evaluate the behavioral disorder in children with GTS. We found that the behavioral disorders include schizoid or anxious, depressed, un- communicative, obsessive-compulsive, somatic complaint, so- cial withdrawal, hyperactive, aggressive, and delinquent behav- ioral syndromes. The abnormal rate is 75%. Among behavioral disorder syndromes, obsessive-compulsive is highest. The P300 latency in children with GTS appeared to be more prolonged than that in normal children; the abnormal rate is 56%. Our result was analyzed by multiple liner regression. The P300 latency has a perfect positive correlation with the obsessive- compulsive syndrome. The P300 latency compared to A-CBCL showed 81% in agreement with the independent assessment of a behavioral disorder. A conclusion of our study can be drawn that a combination of A-CBCL and P300 latency is sensitive in the evaluation of behavioral disorders in children with GTS; the P300 latency may be the preferable and objective one.

193. SIGNIFICANCE AND MEASUREMENT OF MYELIN BASIC PROTEIN ANTIBODY IN CSF FROM CHILDREN WITH CNS DISEASES Chen Ling and Wang Shu-xue, Changchun, China

Myelin basic protein (MBP) as an antigen was extracted from normal human brain white tissue. The level of MBP antibody (MBP-Ab) in CSF was measured with enzyme-linked immuno- sorbent assay in children with CNS diseases. In Guillain-Barr6 syndrome (GBS) the level of MBP-Ab was significantly higher than normal controls (P < .01). So it is useful in the diagnosis of GBS and contributes to the assessment of the GBS course. The study also showed that the level of MBP-Ab in children with tuberculous meningitis was significantly higher than in purulent meningitis, viral encephalitis, and a normal control group. The conjecturing measurement of MBP-Ab in CSF could be a refer- ence index for the differential diagnosis of TB meningitis.

194. PROGRESSIVE IDIOPATHIC LEVODOPA-RE- SPONSIVE DYSTONIA WITH DIURNAL FLUCTUA- TION Johan F. Schoeman, Cape Town, South Africa

Since the description of the syndrome of "hereditary progressive dystonia with marked diurnal fluctuation" by Segawa et al., sporadic cases have been reported. We report the first case of Segawa-type dystonia from the African continent and demon- strate the typical diurnal fluctuation of symptoms and the prompt response to levodopa by means of a video recording.

The patient is a 5-year-old boy of mixed race of unrelated par- ents. Birth and developmental milestones were normal and his medical history, including family history, was negative. At the age of 21/2 years he presented at a teaching hospital with an unsteady gait of 6 months duration. On examination he was found to "go stifP' when walking and tended to invert his left foot. Gowers' sign was positive. The formal neurologic examin- ation, however, was completely normal and a provisional diag- nosis of a "myopathy" was made. Blood tests (including CBC, Astrup, lactate, pyruvate, liver function tests, CK), urine (amino and organic acids), CSF (chemistry and culture), electrophysio- logic tests (EEG, VER, ERG, EMG, nerve conduction) and neu- roimaging (cranial CT, spinal MRI) were all negative. A muscle biopsy showed nonspecific changes. The patient's gait deterior- ated over the next 21/2 years and when first seen by us at age 5 years he could still walk a few steps in the morning but not at all by late afternoon. Walking was characterized by progressively shorter steps and increased tone of the legs, which invariably led to falling over. Apart from appearing depressed, the formal neu- rologic examination was normal. Treatment was started with Sinemet (187 mg levodopa and 18.7 mg carbidopa) and within one day the patient was able to walk and even regained the ability to run. A striking improvement of mood also occurred. Reference: [1] Segawa M, Hosaka A, Miyagawa, et al. Hered- itary progressive dystonia with marked diurnal fluctuation. Adv Neurol 1976;14:215-33.

195. CORPOCALLOSOTOMY IN INTRACTABLE EPI- LEPSY Ledia Troncoso, Fernando Novoa, Arturo Zuleta, and Sergio Valenzuela, Santiago, Chile

About one-quarter of epileptic patients cannot achieve an ade- quate control of seizures, even when available polytherapy is administered. Several surgical methods have been proposed as an alternative treatment in some patients. Central corpocalloso- tomy has proved to be useful in controlling generalized atonic, tonic, and tonic-clonic seizures. Although reports are mainly referred to intellectually normal subjects, recent data suggest benefits in mentally retarded patients as well. We report the results of central corpocallosotomy in 16 patients (9 girls, 7 boys) with a mean age of 12.4 years (S.D.: 4.2), suffering from mental retardation and intractable epilepsy. All had daily atonic episodes (X: 9.5, S.D.: 7.4) and 2-6 other types of seizures, for 3-5 years, and which markedly affected their quality of life. The follow-up of these patients, with a mean of 20.4 months (S.D.: 7.6, range: 8-34) after surgical procedure was performed, shows a highly significant effect on atonic and tonic-clonic seizures, which disappeared in 14 of 16 and 8 of 14 patients, respectively. A significant decrease in tonic, complex partial, myoclonic sei- zures and absence was also observed. At the same time, parents reported improvements in quality of life, expressed as a higher level of autonomy and increased social interaction.

196. OBJECTIVE MEASURE OF TREATMENT OUT- COME IN EPILEPSY Jorge Eslava-Cobos, Bogot& Colombia

Shofer and Temkin [1] presented a proposal for the determina- tion of seizure frequency in terms of time to (X) seizure. Using that general approach we have devised the following formula:

PEDIATRIC NEUROLOGY Vol. 8 No. 5 393